Case 1: 15 year old with flare up of severe ulcerative colitis was found to have significantly raised amylase level (1083 U/L) on routine Azathioprine monitoring. There was no baseline (pre Azathioprine treatment) amylase level recorded. Abdominal pains associated with his flare ups caused diagnostic difficulties with continuation of treatment. He was changed to 6 Mercaptopurine but his hyperamylaesemia continued posing difficulty with clinical management. He had normal imaging of pancreas and normal lipase level.

His paired serum amylase (3703 U/L) and urine amylase (125 U/L) confirmed low urinary amylase. Electrophoresis for amylase iso-enzymes confirmed presence of macroamylase. This helped to restart and continue treatment with Azathioprine, monitoring his amylase level with his previous baseline levels.

Case 2: 6 years old with neuronal migration disorder and intractable epilepsy (on multiple antiepileptic drugs) and gastrostomy feeds was admitted with abdominal pains probably suggesting constipation. Her amylase level was 2846 U/L. She was conservatively managed for possible acute pancreatitis in view of treatment with antiepileptic drugs known to cause pancreatitis. Her amylase level widely fluctuated with no correlation to clinical status. Her imaging and serum lipase level was normal.

Her amylase iso-enzymes electrophoresis suggested rise in predominantly salivary amylase (see table below). She had background of excessive salivation with recent injection of botulinum toxin in to her submandibular gland had not helped her.

Discussion In both these cases, isoamylase electrophoresis helped to establish underlying diagnosis and avoided alteration to the ongoing treatment in view of raised amylase level of doubtful clinical correlation.

Raised amylase level is seen frequently in complex patients treated with multiple medications and is attributed to acute pancreatitis. Due to aetiological uncertainty of acute pancreatitis, this often leads to unnecessary modifications to the treatment. Imaging pancreas, checking lipase and estimating urinary amylase are useful investigations to narrow down differential diagnosis especially when clinical course is unusual. Isoamylase electrophoresis is helpful to look for rise in specific isoenzyme component in these patients to guide long term approach.